PB2318: ACQUIRED WILLEBRAND SYNDROME REVEALING ASYMPTOMATIC MULTIPLE MYELOMA: A CASE REPORT
Authors: W. Chawad, A. Laabichi, Y. Moussadik, Y. chhih, F. ibourk idrissi, A. taouch, S. el fari firdaous, H. talebi, H. hanani, S. belkhettab, M. bouaouad, N. mouatassim, H. khibri, W. ammouri, M. maamar, H. harmouche, Z. tazi mezalek, M. adnaoui
Journal: HemaSphere (2022)

Background: Acquired Willebrand syndrome (AvWS) is a rare hemorrhagic syndrome, clinically and biologically identical to constitutional Willebrand syndrome (vWS). It predominantly affects subjects with no personal or family history of hemorrhage, at an average age of 60. Its prevalence has been estimated by some authors at 0.04%, and over 300 cases have been reported since its discovery in 1968. The pathologies responsible for acquired Willebrand syndrome are diverse; including lymphoproliferative and myeloproliferative syndromes,autoimmune and cardiovascular diseases, solid tumors, and even certain medications. The physiopathology is complex and leads to an accelerated clearance of Willebrand factor. A Willebrand anti-factor antibody is preferentially found during lymphoid proliferation, while the mechanical destruction of high molecular weight multimers is observed during heart problems. Aims: To emphasize the pre-existing link between acquired Willebrand disease and multiple myeloma, as well as show the complexity of this relation. Methods: We report the case of a patient in whom the diagnosis of acquired Willebrand syndrome allowed to reveal an asymptomatic multiple myeloma. Results: A 50-year-old patient was admitted for the etiological assessment of an iron deficiency anemia due to a hemorrhagic symptomatology made up of rectal bleeding and epistaxis. There was no family or personal history of hemorrhage, especially during circumcision and dental extractions. On the biological report, we found microcytic anemia with iron deficiency. The hemostasis assessment revealed an elongated partial thromboplastin time at 2.94 with a correct prothrombine ratio at 100%. Factor XIII has collapsed to 5%. FWV antigen was at 9.4%, and its activity at 3%. This biological profile was in favor of Willebrand disease type 3. On the etiological assessment, protein electrophoresis objectified a monoclonal peak with the presence of an immunoglobulin G kappa at immunofixation. The diagnosis of multiple myeloma was retained due to the presence of 28% of monotypic plasma cells for kappa light chains. CRAB criteria were absent except for anemia which has been attributed to chronic bleeding. ISS score was at 1. FISH was not carried out. The diagnosis of an AVWS on multiple myeloma was retained. Despite the absence of myeloma treatment criteria, the indication for chemotherapy has been made. The patient received 2 courses of chemotherapy combining bortezomib, thalidomide and dexamethasone. The hematological reassessment after 2 cures has shown a partial response on the monoclonal gammopathy. However, the absence of response on Willebrand’s disease was noted: the FWV antigen was at 3% and its activity at 11.5%, which resulted on the interruption of the chemotherapy protocol. The patient was put on rituximab for his von Willebrand’s disease according to the following protocol: 1g on Day 1, and on Day15. The cessation of the hemorrhagic syndrome was noted during the reassessment. Summary/Conclusion: Acquired von Willebrand syndrome is a very heterogeneous hemorrhagic syndrome, usually characterized by mild to moderate hemorrhagic symptoms, which can be severe and life-threatening in some cases. During the investigation of a hemorrhagic syndrome in the elderly, it is crucial to think of the AWVS. We recommend screening for AvWS in patients followed for onco-haematological pathologies when unexplained abnormal bleeding occurs.the treatment of the monoclonal gammapathy can allow the regression of the hemorrhagic manifestations in some cases.